2013, Vol 13, No 3
Mental disorder in systemic lupus erythematosus
AKTUALN NEUROL 2013, 13 (3), p. 168–178
ABSTRACT

The systemic lupus erythematosus (SLE) is a connective tissue disorder, being developed on the basis of the autoallergy. Clinical symptoms of SLE are connected not only with the occurrence of cutaneous exanthemata, but can also manifest itself with the row of symptoms on the part of internal organs, with haematologic distempers and symptoms about the neuropsychiatric character. The pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) is complicated and one tries to become clear her with the production of autoantibodies and with the postponement himself antigen–antibody complexes, what a succession is the cytotoxic damage of neurons, an irregular expression of inflammatory mediators and an influx of inflammatory cells, vascular distempers and an inclination to thrombuses.The entire frequency of the occurrence NPSLE is estimated on 56.3% patients with SLE. Most often mental disorders in patients with lupus erythematosus are mood disorder, anxiety disorder, cognitive disorders and consciousness clouding. An essential aspect there are also drug-induced mental trouble. In the therapeutic approach in the therapy of NPSLE about the slight activity of the disease and/or the slight degree of organ changes complies glucocorticoids in low doses and depending on needs medicines counterphobic, antidepressant, sometimes the valproic acid, the carbamazepine, the lamotrigine and anti-psychotic preparations. In the antithrombotic prophylaxis at ill from the risk population the acetylsalicylic acid complies.

Keywords: systemic lupus erythematosus, neuropsychiatric systemic lupus erythematosus, mental disorders, mood disorders, cognitive disorders
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Rare intramedullary localization of epidermoid cyst – case report
AKTUALN NEUROL 2013, 13 (3), p. 179–182
ABSTRACT

Authors present a case of 54-year-old female treated for intramedullary epidermoid cyst. Patient complained of atypical pain and dysesthesia in lumbosacral region for many years. Patient’s complains increased before admission and were unbearable. Performed clinical and imaging examination revealed intramedullary tumour located in the terminal cone at the level of L2 vertebra. The patient underwent surgical treatment with total tumour removal. Histological examination confirmed epidermoid cyst. Intramedullary location of epidermoid cyst is very rare and constitutes of about 0.7% of all spinal tumours. Due to compression on nervous structures surgical treatment of epidermoid cyst is a method of choice. Authors discuss signs, clinical history, treatment and its results as well as histological findings.

Keywords: intramedullary, intraspinal, spinal cord, tumour, cyst, atypical spinal pain
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Zonisamide – not only antiepileptic drug
AKTUALN NEUROL 2013, 13 (3), p. 183–188
ABSTRACT

Zonisamide – a new generation antiepileptic drug – features a multidirectional mechanism of action. The antiepileptic effect of zonisamide involves blockade of voltage-dependent Na+ and Ca2+ T-type channels, which have a most important role in membrane excitability, suggesting that zonisamide disrupts neuronal synchronized firings and epileptic activity resulting in a limitation of initiation or propagation of seizure. As an anticonvulsant it is used in the treatment of partial and generalized seizures mixed with adults and children, both in combination and in monotherapy. Zonisamide has also been approved for the treatment of Parkinson’s disease. In several double-blind, randomized, multicentre trials, carried out on Parkinson’s disease patients, who showed insufficient response to L-dopa treatment, zonisamide significantly improved motor functions determined by beneficial changes in the total score of the Unified Parkinson’s Disease Rating Scale (UPDRS). It is known that the drug increases dopaminergic transmission. New research shows that both, dopaminergic and non-dopaminergic mechanisms could equally be involved. Many authors emphasizes the importance neuroprotective activity of zonisamide. However, there are still some aspects that need to be explained in further studies. Clinical trials are conducted to evaluate its efficacy in bipolar disorder, neuropathic pain and migraine, anxiety disorders, eating disorders and in patients with alcohol dependence. Results of the studies are promising and provide a new possibilities of zonisamide application. However, there is a strong need to expand research in this area.

Keywords: zonisamide, mechanism of action, drug safety, indications, new directions for zonisamide use
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Application of mobilization technique during subacromial impingement syndrome therapy
AKTUALN NEUROL 2013, 13 (3), p. 189–194
ABSTRACT

Background: A shoulder’s pain becomes more and more popular affection. They affect young just as older people. Young people are exposed for quite frequent, forced accepting a sitting position at work, but also for injuries associated with practicing careless physical activity. In turn, for elder people a summing up of overloads over a distance area of the entire life. There are many causes of the painful shoulder. Scientific sources are announcing that about 40–60% of all kinds of shoulder pains are caused by subacromial impingement syndrome. Subacromial impingement syndrome is an articulation’s dysfunction. The study objective was to assess the effectiveness of mobilization technique during subacromial impingement syndrome therapy. Material and methods: The study group consisted of 30 patients who were diagnosed by physiotherapeutists and orthopaedists – a subacromial impingement syndrome. The study group account for both female and male sex. The patients were directed to rehabilitation centre for two weeks procedures program. During rehabilitation, patients had a humeral joint’s mobilization. A visual analogue scale and a modified Laitinen’s questionnaire of pain assessment was used in the research. Both scales were used before and after a rehabilitation program. The Commission of Bioethics’s permission was approved to carry out the study. Results: The common principles of treatment of the patients with subacromial impingement syndrome were: a reduction of pain level, an increase of movement extent in humeral joint and a reduction of pain frequency compared with position before rehabilitation. Moreover, after rehabilitation, patients applied reduced number of analgesics. Only two patients, in spite of intensively conducted rehabilitation program, did not get better improvement of health condition. Conclusions: An application of mobilization techniques in the treatment of subacromial impingement syndrome is an effective method improving the quality of patients’ life. The introduction of mobilization techniques in the rehabilitation program of subacromial impingement syndrome helps to reduce the intensity of pain. It is also a beneficial treatment that affects the increased range of motion in the joint. Moreover, it helps patients to reduce the amount of medication.

Keywords: subacromial impingement syndrome, mobilization, shoulder, rehabilitation procedures, pain
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Creutzfeldt-Jakob disease − diagnostic methods
AKTUALN NEUROL 2013, 13 (3), p. 195–200
ABSTRACT

Creutzfeldt-Jakob disease (CJD) belongs to prion diseases (transmissible spongiform encephalopathies). It’s a progressive, fatal and untreatable neurodegenerative disease. Creutzfeldt-Jakob disease is caused by the pathological prion protein (scrapie, PrPSc) that accumulates in the central nervous system and other tissues. Symptoms of the disease include: rapidly progressing dementia, speach impairment and blurred vision, involuntary muscle movements e.g. myoclonus, ataxia, paresis or problems of balance and co-ordination. The direct cause of death is mostly pneumonia. The incubation period may vary from a few months to several years, death occurs over a few weeks or months after the onset of clinical symptoms. According to the current criteria, histopathological verification is mandatory for definitive diagnosis for a CJD. The neuropathological features of CJD are characterized by a triad of spongiosis, astrocytosis and neuronal loss. It’s difficult to diagnose CJD because of the lack of a biospecific marker for CJD and not always characteristic clinical signs, which require differentiation with other disorders associated with dementia. This article is a short review of the recently published data on detection methods.

Keywords: prion, prion disease, diagnosis, Creutzfeldt-Jakob disease, transmissible spongiform encephalopathies
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Clinical aspects of prion diseases
AKTUALN NEUROL 2013, 13 (3), p. 201–207
ABSTRACT

Human prion disease is divided into three broad classes: idiopathic, genetic and acquired, reflecting different causation with resulting variations in clinical and neuropathological features. There are significant differences in clinical presentation both between and within these three groups, but all are progressive, fatal brain diseases with dementia, cerebellar ataxia and involuntary movements being particularly prominent features. Absolutely definite diagnosis requires neuropathological analysis of brain tissue (via cerebral biopsy in life or at autopsy) but there are established clinical diagnostic criteria and a variety of supportive investigations including abnormalities in the EEG, cerebral MRI, PRNP genetic analysis and CSF protein analysis. The precise results of these supportive investigations and their diagnostic utility vary somewhat depending on the type of prion disease. For example, EEG periodic discharges are a characteristic finding in sporadic but not variant CJD, for variant CJD, tonsil biopsy is an additional test and, in genetic prion disease, blood testing is possible for pathogenic PRNP mutations.

Keywords: prion diseases, clinical aspects of prion diseases, Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, iatrogenic Creutzfeldt-Jakob disease
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Detection of prion protein in body fluids: new perspectives in Creutzfeldt-Jakob disease diagnostics
AKTUALN NEUROL 2013, 13 (3), p. 208–216
ABSTRACT

The diagnostics of prion diseases, including Creutzfeldt-Jakob disease (CJD), is still challenging as none of currently available tests, including magnetic resonance imaging, electroencephalogram and detection of 14-3-3 protein in cerebrospinal fluid, are sufficient for definite premortem diagnosis. This paper presents sensitive methods based on the ability of PrPSc protein to induce the PrPc to PrPSc conversion in vitro which have been developed within the last few years in order to detect trace amounts of the abnormal prion protein in cerebrospinal fluid or blood. Among those methods, the quaking-induced conversion (QuIC) with the use of purified recombined PrPc protein as a substrate seems to be the most promising. We discuss the current research in optimization of the method as well as the perspectives of its possible applications in diagnostics and scientific investigations.

Keywords: Creutzfeldt-Jakob disease, diagnostic markers, prion protein amplification, protein misfolding cyclic amplification (PMCA), quaking-induced conversion (QuIC)
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Kuru, the first prion disease: a travel back in time from Papua New Guinea to Neanderthals extinction
AKTUALN NEUROL 2013, 13 (3), p. 217–229
ABSTRACT

Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the XX century. “Kuru” in the Fore language of Papua New Guinea means to tremble from fever or cold. Kuru was restricted to natives of the Fore linguistic group in Papua New Guinea’s Eastern Highlands and neighbouring linguistic groups. Ritualistic endocannibalism (eating of relatives as part of a mourning ritual in contrast to eating enemies, i.e. exocannibalism) was practiced not only in the kuru area but in many surrounding Eastern Highland groups in which kuru never developed. The first who formally published the hypothesis that kuru spreads through cannibalism was Lindenbaum and Glasse.

Keywords: kuru, choroby wywoływane przez priony, priony, neuropatologia, D. Carleton Gajdusek
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Amyloids and prionoids – is there a time to be afraid?
AKTUALN NEUROL 2013, 13 (3), p. 230–239
ABSTRACT

The new era has come to microbiology as we have realized that the unconventional viruses of kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), scrapie, and bovine spongiform encephalopathy (BSE) are infectious amyloid proteins and that these transmissible spongiform dementias are brain amyloidoses. This quotation from a Nobel laureate, D. Carleton Gajdusek, illustrates the best the content of this paper. Amyloid is a generic term, which embraces the fibrillary cross-β-sheet quaternary structure of any protein. All amyloids, irrespective of their amino acid sequences, are formed through nucleation/polymerization reactions in which oligomeric structures (small aggregates) composed of a limited number of a given protein moiety (a seed) nucleates other moieties. As a result, the β-pleated secondary structure predominates. Such proteins are called “prionoids” as opposed to “real” prions, which are infectious, or transmissible, in a microbiological sense; they spread between individuals and cause macro-epidemics, such as kuru, BSE and iatrogenic CJD. In this review, prions and prionoids, and their interrelatedness, will be discussed.

Keywords: prions, prionoids, amyloids, nucleation, oligomers
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